Background Homozygous familial hypercholesterolaemia (hoFH) is a rare inherited disorder characterised by extreme elevation of low-density lipoprotein (LDL)-cholesterol, accelerated coronary artery disease (CAD) and premature death. Aggressive LDL-cholesterol lowering therapies are important for survival but these are not available worldwide. Objective To compare and contrast cardiovascular outcomes and mortality of hoFH patients in two countries with disparate use of lipoprotein apheresis (LA) and modern therapies for lowering LDL-cholesterol. Methods A retrospective study was undertaken comparing cardiovascular disease (CVD)-free survival and mortality in 44 hoFH patients who were treated statins but not LA, from a centre in Beijing, China, and 18 hoFH patients who were treated with LA and novel therapies from an early age, from a centre in Rome, Italy. Results CVD-free survival and survival was significantly reduced in the Chinese patients compared with the Italian patients after 30 years of follow-up (log-rank p<0.01). In a pooled analysis, cardiovascular survival was significantly increased with earlier age at treatment, longer duration of treatment and lower on-treatment LDL-cholesterol concentrations (p<0.05). Additionally, the probability of a CVD event and death were increased in patients that carried a null mutation in the LDLR or had elevated lipoprotein(a). Conclusions We show that CAD outcomes in patients with hoFH can be significantly improved with earlier and potent LDL-cholesterol lowering with pharmacotherapies and LA. This has major implications for countries, such as China, where the models of care for hoFH remains underdeveloped.